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MABN2280-25UL Anti-FMR1polyG Antibody, clone 8FM-2F7

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MABN2280-25UL
25 µL  
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      Replacement Information

      Tabella delle specifiche principali

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HIF, IH(P), WBMAscitesMonoclonal Antibody
      Description
      Catalogue NumberMABN2280-25UL
      DescriptionAnti-FMR1polyG Antibody, clone 8FM-2F7
      Alternate Names
      • Synaptic functional regulator FMR1
      • Fragile X mental retardation protein 1
      • FMRP
      • Protein FMR-1
      Background InformationSynaptic functional regulator FMR1 (UniProt: Q06787; also known as Fragile X mental retardation protein 1, FMRP, Protein FMR-1) is encoded by the FMR1 gene (Gene ID: 2332) in human. FMR-1 is a multifunctional polyribosome-associated RNA-binding protein that plays a central role in neuronal development and synaptic plasticity through the regulation of alternative mRNA splicing, mRNA stability, mRNA dendritic transport and postsynaptic local protein synthesis of a subset of mRNAs. It plays a role in mRNA nuclear export and together with export factor NXF2, it is involved in the regulation of the NXF1 mRNA stability in neurons. It is also reported to stabilize the scaffolding postsynaptic density protein DLG4/PSD-95 and the myelin basic protein mRNAs in hippocampal neurons and glial cells, respectively and this stabilization is further increased in response to metabotropic glutamate receptor stimulation. A CGG repeat expansion (55-200) in the 5 UTR of FMR1 gene is reported to cause Fragile X-associated Tremor Ataxia Syndrome (FXTAS). The expanded CGG repeat is associated with elevated FMR1 mRNA expression that results and neurodegeneration. The CGG repeat expansion triggers repeat-associated non-ATG-initiated translation (RAN or RANT) within the 5 UTR of FMR1 mRNA, resulting in the production of fusion proteins (FMR1polyG) that contain N-terminal polyglycine fused to either C-terminal FMR1 in-frame sequence or one of two FMR1 frame-shift sequences. The FMR1polyG-positive aggregates closely resemble neuronal intranuclear inclusions seen in polyglutamine diseases and other protein-mediated neurodegenerative disorders. (Ref.: Buijsen, RAM., et al. (2014). Acta Neuropathol. Commun. 2:162; Todd, PK et al. (2013). Neuron. 78(3); 440-455).
      References
      Product Information
      FormatAscites
      PresentationMouse monoclonal antibody in ascites without preservatives.
      Applications
      ApplicationAnti-FMR1polyG, clone 8FM-2F7, Cat. No. MABN2280, is a highly specific mouse monoclonal antibody that targets Synaptic functional regulator FMR1 (FMR1polyG) and has been tested for use Immunofluorescence, Immunohistochemistry (Paraffin), and Western Blotting.
      Key Applications
      • Immunofluorescence
      • Immunohistochemistry (Paraffin)
      • Western Blotting
      Application NotesWestern Blotting Analysis: A 1:500 dilution from a representative lot detected FMR1polyG in lysate from HEK293 cells overexpressing FMR1polyG.

      Immunohistochemistry Analysis: A representative lot detected FMR1polyG in Immunohistochemistry applications (Buijsen, R.A., et. al. (2014). Acta Neuropathol Commun. 2:162; Sellier, C., et. al. (2017). Neuron. 93(2):331-347).

      Western Blotting Analysis: A representative lot detected FMR1polyG in Western Blotting applications (Sellier, C., et. al. (2017). Neuron. 93(2):331-347).

      Immunohistochemistry Analysis: A 1:50 dilution from a representative lot detected FMR1polyG in human cerebral cortex and human testis tissues.

      Immunofluorescence Analysis: A representative lot detected FMR1polyG in Immunofluorescence applications (Buijsen, R.A., et. al. (2014). Acta Neuropathol Commun. 2:162; Sellier, C., et. al. (2017). Neuron. 93(2):331-347).
      Biological Information
      ImmunogenOvalbumin-conjugated linear peptide with EAPLPGGVRQRGG sequence. Note: This protein is translated within the 5'UTR of the FMR1 gene and in a different frame resulting in a protein with different sequence and containing polyGlycine.
      EpitopeN-terminus
      Clone8FM-2F7
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      SpecificityClone 8FM-2F7 specifically detects FMR1polyG in human cells.
      IsotypeIgG1κ
      Species Reactivity
      • Human
      Species Reactivity NoteHuman.
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • FMR1
      Purification MethodUnpurified
      UniProt Number
      Molecular Weight~35 kDa observed. Uncharacterized bands may be observed in some lysate(s).
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in HEK293 cell lysates expressing FMR1polyG.

      Western Blotting Analysis: A 1:500 dilution of this antibody detected FMR1polyG in HEK293 cell lysates expressing FMR1polyG.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at -20°C from date of receipt. Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.
      Packaging Information
      Material Size25 µL
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Numero di catalogo GTIN
      MABN2280-25UL 04054839460029